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Introduction: Sickle cell anemia is a genetic disorder commonly seen in rural population of western and central India. Being an important cause of hemolytic anemia, repeated blood transfusions are frequently required for the sickle cell anemia cases. Few studies have exclusively addressed hematological profile of sickle cell disease patients. This study was planned to study the hematological profile of children with special reference to body iron stores. Methods: This was a prospective observational study carried out in department of pediatrics S.B.K.S MIRC. All the confirmed cases of children diagnosed with sickle cell disease or sickle β thalassemia were subjected to complete hematological analysis in special reference to body iron store by evaluation of serum iron, TIBC, % transferrin saturation and serum ferritin to find out total body iron store. Results: About 110 patients were included in the study. In our study low MCV values were seen in 60.4% of patients, low MCH values in 84.9% of patients while MCHC was normal in 94.3% of patients. Serum iron levels showed normal values in majority of patients (94.3%) while transferrin saturation was low in 62.3% patients thus showing the discrepancy on correlating both values for diagnosis of iron deficiency. Conclusion: Out of various parameters for diagnosis of iron status in SCD-SS patients, low MCV, MCH, Serum iron level and transferrin saturation more consistently associated with iron deficient state.

Background: Double outlet right ventricle (DORV) with two well-developed ventricles and with a remote ventricular septal defect (VSD) may present a therapeutic challenge. Echocardiographic imaging of such complex cases does not always provide all of the information required to decide on an operative approach (biventricular or univentricular) and to design an intracardiac baffle to direct left ventricular outflow through the VSD and to the aorta for biventricular repair. A three dimensional (3D) printed model of the heart based upon data derived from computed tomography (CT) or magnetic resonance imaging (MRI) may contribute to a more complete appreciation of the intracardiac anatomy. Methods: From April to September 2015, six consecutive patients with DORV and remote VSD underwent CT/MRI scans. Data sets from these studies were used to generate life-size 3D models using a 3D printer. We compared the assessment of 3D printed heart model findings with information obtained from echocardiography, CT, or cardiac MRI and with details of the surgeon’s intraoperative direct observations when available. Quantification of the information provided by the 3D model was achieved using a unique scale that was created for the purpose of this study. The accuracy and utility of information derived preoperatively from the models were assessed. Results: Six data sets from six patients were analyzed. Five data sets could be successfully used to create sandstone models using 3D printing. The five patients ranged from 7 months to 11 years of age and weighed 6.7 to 26 kg. The spatial orientation of the heart in the thorax, the relationships of the great arteries and the semilunar valves, the size and location of the VSD were well appreciated in all models, as were the anticipated dimensions and orientation of a surgically planned interventricular baffle. Three of the five patients underwent successful biventricular repair. Conclusion: The 3D printed models scored higher than conventional imaging, with respect to most aspects of the surface spatial orientation and intracardiac anatomy. The models are a useful adjunct in preoperative assessment of complex DORV. The unique scale helps quantify the advantages and limitations of the 3D heart models.

Hydroxyurea, a myelosuppressive agent, is the only effective drug proven to reduce the frequency of painful episodes. It raises the level of HbF and the haemoglobin level. It usually decreases the rate of painful episodes by 50 %. It was first tested in sickle cell disease in 1984. It also decreases the rate of ACS episodes and blood transfusions by ~50 % in adults. It was developed as an anticancer drug and has been used to treat myeloproliferative syndromes-leukemia, melanoma, and ovarian cancer. It was approved for use by FDA in adults. Side effects includes neutropenia, bone marrow suppression, elevation of hepatic enzymes, anorexia, nausea, vomiting and infertility.

Kawasaki disease (KD) is associated with coronary artery lesion, especially in the form of aneurysm and ectasia. The first case is 1-year, 9-month-old female; the second is a 13-month-old male, the third is a 14-month-old female, and the fourth is a 5-year, 6-month-old male who all satisfied the criteria of KD. Echocardiography done for all the series showed dilatation of the left anterior descending coronary artery measuring 2.15 mm with Z score of 2.53 and focal dilatation of left main coronary artery with maximum diameter of 2.9 mm with Z score of 2.44 for the first case, prominent left coronary artery measuring 2.19 mm with Z score of 1.28 with borderline dilatation of the circumflex coronary artery measuring 2 mm with a Z score of 2.13 for the second case, and left coronary dilatation measuring 3.5 mm with Z score of 3.61 and borderline left coronary artery dilatation measuring 3.5 mm with a Z score of 2.24, respectively, for the last two cases. We documented four cases of KD as much as we know, and all the four cases presented with coronary artery aneurism with a good response to chemotherapy.

Rhabdomyomas are rare tumors in children often associated with tuberous sclerosis. They are usually diagnosed during antenatal period or as an incidental finding. The first case is a 2.5-month-old child who was diagnosed to have rhabdomyomas at gestational age of 24 weeks. Electrocardiogram revealed left axis deviation and multiple premature atrial contractions. Echocardiography showed massive tumor occupying the right ventricular cavity. Tuberous sclerosis was seen on magnetic resonance imaging. He was commenced on everolimus with obvious regression of tumor. The second case is an 8-month-old male who was referred to us for cardiac evaluation. Echocardiography showed two masses of rhabdomyoma. He was also placed on everolimus but was lost to follow-up. Rhabdomyomas are rare tumors of the heart which respond well to chemotherapy.